D8043
Anti-Dystrophin Antibody
mouse monoclonal, MANDRA1
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| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| 0.2 mL | Available to ship TODAYfromMILWAUKEE | $568.00 |
About This Item
Conjugate:
unconjugated
Clone:
MANDRA1, monoclonal
Application:
ARR, IF
Citations:
29
$568.00
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A recombinant, preservative-free antibody is available for your target. Try ZMS1051
Product Name
Monoclonal Anti-Dystrophin antibody produced in mouse, clone MANDRA1, ascites fluid
biological source
mouse
Quality Level
conjugate
unconjugated
antibody form
ascites fluid
antibody product type
primary antibodies
clone
MANDRA1, monoclonal
contains
15 mM sodium azide
species reactivity
rat, human, mouse, fish
technique(s)
indirect immunofluorescence: 1:100 using freshly dissected and frozen human or animal muscle tissue., microarray: suitable
isotype
IgG1
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... DMD(1756)
mouse ... Dmd(13405)
rat ... Dmd(24907)
General description
Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse. Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of DMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine. The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies.
The C-terminal domain of the human dystrophin molecule (amino acids residues 3558-3684) is present in normal muscle tissue. It is also present in nearly all Becker muscular dystrophies, but is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx).
The antibody recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (amino acid residues 3558-3684). Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers. By immunoblotting, the antibody stains dystrophin (427 kDa) in muscle and brain extracts. It also stains the 70-75 kDa protein known as Apo-Dystrophin-1 or Dp71.4 This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma). The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding. The antibody exhibits a wide interspecies cross-reactivity. It is useful in ELISA and capture ELISA. The antibody is specific to dystrophin and does not react with α-actinin and utrophin, an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP).
Immunogen
fusion protein containing the C-terminal amino acids of human dystrophine.
Application
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Immunofluorescence (1 paper)
Western Blotting (1 paper)
Immunofluorescence (1 paper)
Western Blotting (1 paper)
Mouse monoclonal clone MANDRA1 anti-Dystrophin antibody may be used for the localization of dystrophin using various immunochemical assays such as ELISA, capture ELISA, immunoblot, and immunohistochemistry. Monoclonal antibodies against defined regions of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.
Biochem/physiol Actions
The antibody recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (amino acid residues 3558-3684). Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers. By immunoblotting, the antibody stains dystrophin (427 kDa) in muscle and brain extracts. It also stains the 70-75 kDa protein known as Apo-Dystrophin-1 or Dp71.4 This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma). The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding. The antibody exhibits a wide interspecies cross-reactivity. It is useful in ELISA and capture ELISA. The antibody is specific to dystrophin and does not react with α-actinin and utrophin, an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP).
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
1 of 1
This Item | |||
|---|---|---|---|
| Gene Information human ... DMD(1756) | Gene Information human ... DMD(1756) | Gene Information human ... DMD(1756) | Gene Information human ... DMD(1756) |
| conjugate unconjugated | conjugate - | conjugate unconjugated | conjugate - |
| clone MANDRA1, monoclonal | clone MANDRA1, monoclonal | clone MANDYS8, monoclonal | clone MANDYS8, monoclonal |
| antibody form ascites fluid | antibody form purified from hybridoma cell culture | antibody form ascites fluid | antibody form purified from hybridoma cell culture |
| biological source mouse | biological source mouse | biological source mouse | biological source mouse |
| species reactivity rat, human, mouse, fish | species reactivity zebrafish, rat, mouse, human | species reactivity chicken, rat, human, pig, rabbit, mouse | species reactivity porcine, rabbit, mouse, rat, human |
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Storage Class
13 - Non Combustible Solids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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Related Content
Datasheet
Jérome Fleuriet et al.
Scientific reports, 10(1), 11927-11927 (2020-07-19)
The ability of sustained treatment of a single extraocular muscle with glial cell line-derived neurotrophic factor (GDNF) to produce a strabismus in infant non-human primates was tested. Six infant non-human primates received a pellet containing GDNF, releasing 2 µg/day for
Michelle Goody et al.
PLoS currents, 9 (2017-12-01)
Both genetic and infectious diseases can result in skeletal muscle degeneration, inflammation, pain, and/or weakness. Duchenne muscular dystrophy (DMD) is the most common congenital muscle disease. DMD causes progressive muscle wasting due to mutations in Dystrophin. Influenza A and B
Tomonari Awaya et al.
PloS one, 7(12), e51638-e51638 (2012-12-14)
Human embryonic stem (ES) cells and induced pluripotent stem (iPS) cells are promising sources for the cell therapy of muscle diseases and can serve as powerful experimental tools for skeletal muscle research, provided an effective method to induce skeletal muscle
Global Trade Item Number
| SKU | GTIN |
|---|---|
| D8043-.2ML | 04061837720192 |
| D8043-.5ML | 04061837720208 |